Paediatric cutaneous lymphomas including rare subtypes: A 40-year experience at a tertiary referral centre

Silvia Alberti-Violetti; Gianluca Avallone; Cristiana Colonna; Gianluca Tavoletti; Luigia Venegoni; Valentina Merlo; Stefano Cambiaghi; Angelo V Marzano; Emilio Berti; Riccardo Cavalli

doi:10.1111/jdv.20028

Paediatric cutaneous lymphomas including rare subtypes: A 40-year experience at a tertiary referral centre

J Eur Acad Dermatol Venereol. 2024 Apr 23. doi: 10.1111/jdv.20028. Online ahead of print.

Authors

Silvia Alberti-Violetti^{1

2}, Gianluca Avallone^{1

2

3}, Cristiana Colonna⁴, Gianluca Tavoletti^{1

2}, Luigia Venegoni², Valentina Merlo², Stefano Cambiaghi⁴, Angelo V Marzano^{1

2}, Emilio Berti^{1

5}, Riccardo Cavalli⁴

Affiliations

¹ Dermatology Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
² Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy.
³ Dermatology Clinic, Department of Medical Sciences, University of Turin, Turin, Italy.
⁴ Pediatric Dermatology Unit, Department of Clinical Sciences and Community Health, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
⁵ Inter-Hospital Pathology Division, IRCCS MultiMedica, Milan, Italy.

PMID: 38650545
DOI: 10.1111/jdv.20028

Abstract

Background: Primary cutaneous lymphomas are neoplasms of the immune system with a distinct tropism for the skin and an absence of extracutaneous manifestations at the time of diagnosis. Studies focusing on cutaneous lymphomas in children and adolescents remain scarce and often do not encompass the rare subtypes.

Objectives: To address this knowledge gap by describing the clinical, histological and molecular characteristics of a large group of paediatric patients affected by primary cutaneous lymphoma. We also provided the Paediatric Primary Cutaneous Lymphoma Atlas that illustrates the clinicopathological spectrum of observed presentations, in the hope of supporting other physicians in the diagnostic process.

Methods: Retrospective chart review of paediatric patients diagnosed with primary cutaneous lymphomas between 1980 and 2022 at the Paediatric Dermatology Unit of Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan.

Results: A total of 101 patients (58 males, 43 females) met the inclusion criteria. The most common subtypes were lymphomatoid papulosis (n = 48) and mycosis fungoides (n = 31). These were followed by primary cutaneous CD4⁺ small/medium T-cell lymphoproliferative disorders (n = 7), primary cutaneous anaplastic large-cell lymphomas (n = 5), primary cutaneous marginal zone B-cell lymphomas (n = 3), primary cutaneous follicle centre lymphomas (n = 2), subcutaneous panniculitis-like T-cell lymphomas (n = 2), primary cutaneous peripheral T-cell lymphoma not otherwise specified (n = 1), primary cutaneous precursor B-lymphoblastic lymphoma (n = 1) and Sézary syndrome (n = 1). Clinical follow-up data covering a median of 70.8 months (range 1-324) were available for 74 patients, of whom three died due to cutaneous lymphoma.

Conclusions: Our findings shed light on the peculiar aspects and long-term outcomes of paediatric cutaneous lymphomas, particularly emphasizing their distinctive features in comparison to their adult counterparts and exploring the less common subtypes. Further larger-scale studies are warranted to better characterize these entities and to achieve a more rapid and accurate diagnosis.